Management of Inherited Arrhythmia Syndromes: A HiRO Consensus Handbook on Process of Care
Mikyla L. Janzen, MSc,
Brianna Davies, MSc,
Zachary W.M. Laksman, MD,
Jason D. Roberts, MD,
Shubhayan Sanatani, MD,
Christian Steinberg, MD,
Rafik Tadros, MD,
Julia Cadrin-Tourigny, MD,
Ciorsti MacIntyre, MD,
Joseph Atallah, MD,
Anne Fournier, MD,
Martin S. Green, MD,
Robert Hamilton, MD,
Habib R. Khan, MBBS, PhD,
Shane Kimber, MD,
Steven White, MD, PhD,
Jacqueline Joza, MD,
Bhavanesh Makanjee, MD,
Erkan Ilhan, MD,
David Lee, MD,
Simon Hansom, MD,
Alexios Hadjis, MD,
Laura Arbour, MD,
Richard Leather, MD,
Colette Seifer, MD,
Paul Angaran, MD,
Christopher S. Simpson, MD,
Jeffrey S. Healey, MD,
Martin Gardner, MD,
Mario Talajic, MD,
Andrew D. Krahn, MD
Affiliations
Mikyla L. Janzen, MSc
Centre for Cardiovascular Innovation, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada
Brianna Davies, MSc
Centre for Cardiovascular Innovation, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada
Zachary W.M. Laksman, MD
Centre for Cardiovascular Innovation, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada
Jason D. Roberts, MD
Population Health Research Institute, Hamilton, Ontario, Canada
Shubhayan Sanatani, MD
Children’s Heart Centre, BC Children’s Hospital, Vancouver, British Columbia, Canada
Christian Steinberg, MD
Institut Universitaire de Cardiologie et Pneumologie de Quebec, Laval University, Quebec City, Quebec, Canada
Rafik Tadros, MD
Cardiovascular Genetics Centre, Montreal Heart Institute and Faculty of Medicine, Université de Montréal, Montreal, Quebec, Canada
Julia Cadrin-Tourigny, MD
Cardiovascular Genetics Centre, Montreal Heart Institute and Faculty of Medicine, Université de Montréal, Montreal, Quebec, Canada
Ciorsti MacIntyre, MD
Department of Cardiovascular Medicine, Division of Heart Rhythm Services, Windland Smith Rice Genetic Heart Rhythm Clinic, Mayo Clinic, Rochester, Minnesota, USA
Joseph Atallah, MD
Division of Cardiology, Department of Pediatrics, University of Alberta Stollery Children’s Hospital, Edmonton, Alberta, Canada
Anne Fournier, MD
Division of Pediatric Cardiology, CHU Sante-Justine, Universite de Montreal, Montreal, Quebec, Canada
Martin S. Green, MD
University of Ottawa Heart Institute, Ottawa, Ontario, Canada
Robert Hamilton, MD
Division of Cardiology, The Hospital for Sick Children (SickKids), Toronto, Ontario, Canada
Habib R. Khan, MBBS, PhD
Section of Cardiac Electrophysiology, Division of Cardiology, Department of Medicine, Western University, London, Ontario, Canada
Shane Kimber, MD
Division of Cardiology, Department of Medicine, University of Alberta, Edmonton, Alberta, Canada
Steven White, MD, PhD
Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, British Columbia, Canada
Jacqueline Joza, MD
Division of Cardiology, McGill University Health Centre, Montreal, Quebec, Canada
Bhavanesh Makanjee, MD
Heart Health Institute, Scarborough Health Network, Scarborough, Ontario, Canada
Erkan Ilhan, MD
Libin Cardiovascular Institute, University of Calgary, Calgary, Alberta, Canada
David Lee, MD
Division of Cardiology, QEII Health Sciences Centre, Halifax, Nova Scotia, Canada
Simon Hansom, MD
University of Ottawa Heart Institute, Ottawa, Ontario, Canada
Alexios Hadjis, MD
Division of Cardiology, Hopital du Sacre-Coeur de Montreal, Montreal, Quebec, Canada
Laura Arbour, MD
Royal Jubilee Hospital, Victoria, British Columbia, Canada
Richard Leather, MD
Royal Jubilee Hospital, Victoria, British Columbia, Canada
Colette Seifer, MD
St Boniface Hospital, University of Manitoba, Winnipeg, Manitoba, Canada
Paul Angaran, MD
Unity Health, St Michael’s Hospital, University of Toronto, Toronto, Ontario, Canada
Christopher S. Simpson, MD
Department of Medicine, Queen’s University, Kingston, Ontario, Canada
Jeffrey S. Healey, MD
Population Health Research Institute, Hamilton, Ontario, Canada
Martin Gardner, MD
Division of Cardiology, QEII Health Sciences Centre, Halifax, Nova Scotia, Canada
Mario Talajic, MD
Cardiovascular Genetics Centre, Montreal Heart Institute and Faculty of Medicine, Université de Montréal, Montreal, Quebec, Canada
Andrew D. Krahn, MD
Centre for Cardiovascular Innovation, Division of Cardiology, Department of Medicine, University of British Columbia, Vancouver, British Columbia, Canada; Corresponding author: Dr Andrew D. Krahn, Hearts in Rhythm Organization, Room 220, 1033 Davie St, Vancouver, British Columbia V6E 1M7, Canada. Tel.: +1-604-682-2344 x63260; fax: +1-604-806-9474.
Inherited arrhythmia syndromes are rare genetic conditions that predispose seemingly healthy individuals to sudden cardiac arrest and death. The Hearts in Rhythm Organization is a multidisciplinary Canadian network of clinicians, researchers, patients, and families that aims to improve care for patients and families with inherited cardiac conditions, focused on those that confer predisposition to arrhythmia and sudden cardiac arrest and/or death. The field is rapidly evolving as research discoveries increase. A streamlined, practical guide for providers to diagnose and follow pediatric and adult patients with inherited cardiac conditions represents a useful tool to improve health system utilization, clinical management, and research related to these conditions. This review provides consensus care pathways for 7 conditions, including the 4 most common inherited cardiac conditions that confer predisposition to arrhythmia, with scenarios to guide investigation, diagnosis, risk stratification, and management. These conditions include Brugada syndrome, long QT syndrome, arrhythmogenic right ventricular cardiomyopathy and related arrhythmogenic cardiomyopathies, and catecholaminergic polymorphic ventricular tachycardia. In addition, an approach to investigating and managing sudden cardiac arrest, sudden unexpected death, and first-degree family members of affected individuals is provided. Referral to specialized cardiogenetic clinics should be considered in most cases. The intention of this review is to offer a framework for the process of care that is useful for both experts and nonexperts, and related allied disciplines such as hospital management, diagnostic services, coroners, and pathologists, in order to provide high-quality, multidisciplinary, standardized care. Résumé: Les syndromes d’arythmie héréditaires sont des troubles génétiques rares qui prédisposent des personnes en apparence en bonne santé à un arrêt cardiaque soudain et à la mort. L’organisation Hearts in Rhythm Organization est un réseau multidisciplinaire canadien qui regroupe des cliniciens, des chercheurs ainsi que des patients et leurs proches dans le but d’améliorer les soins prodigués aux patients atteints de maladies cardiaques héréditaires et à leur famille, en particulier dans le cas des maladies qui entraînent une prédisposition à l’arythmie et à un arrêt cardiaque soudain et/ou à la mort. Puisque ce champ de recherche évolue rapidement, la mise au point d’un guide pratique et simple à l’intention des professionnels de la santé pour le diagnostic et le suivi des patients enfants et adultes présentant une maladie cardiaque héréditaire serait donc un outil intéressant pour améliorer l’utilisation du système de santé et la prise en charge clinique de ces maladies tout en orientant la recherche à ce propos. La présente synthèse expose les trajectoires de soins faisant l’objet d’un consensus pour sept maladies, dont les quatre maladies cardiaques héréditaires les plus courantes qui prédisposent à l’arythmie. Elle présente aussi des scénarios pour orienter les examens, le diagnostic, la stratification du risque et la prise en charge des patients. Ces maladies sont le syndrome de Brugada, le syndrome du QT long, la cardiomyopathie arythmogénique du ventricule droit et les cardiomyopathies arythmogènes associées, et la tachycardie ventriculaire polymorphe catécholaminergique. En outre, une approche pour la prise en charge de l’arrêt cardiaque soudain, de mort subite inattendue et des membres de la famille immédiate de la personne touchée est proposée. L’orientation vers des cliniques spécialisées en cardiogénétique doit être envisagée dans la plupart des cas. L’objectif est d'établir un cadre de soins qui soit utile pour les experts et les non-experts ainsi que pour les professionnels des domaines connexes, par exemple le personnel de l’administration hospitalière et des services diagnostiques, les coroners et les pathologistes, en vue d’offrir des soins multidisciplinaires normalisés de grande qualité.
